Biomedical subjects

Renata Langfort

Publications and source records attributed to Renata Langfort.

At least 19 recordsLinked to original sources

PubMed2005

Disseminated Mycobacterium avium infection in a 20-year-old female with partial recessive IFNgammaR1 deficiency.

We report the case of a 20-year-old female with disseminated Mycobacterium avium disease involving bones, lungs and brain. She was completely healthy up until the present illness and had been vaccinated with BCG in infancy without complications. Mycobacteriosis progressed in spite of treatment with antituberculous drugs and was controlled only after addition of interferon-gamma subcutaneously. A homozygous hypomorphic I87T mutation was found in the gene encoding the ligand-binding chain of the IFN-gamma receptor (IFNgammaR1). This mutation is the only known recessive hypomorphic lesion in IFNGR1 and had been reported before in only 1 child with curable BCG infection and his sibling with primary tuberculosis. Our report illustrates the clinical heterogeneity of patients sharing exactly the same form of partial recessive IFNgammaR1 deficiency. A diagnosis of partial recessive IFNgammaR1 deficiency should be contemplated in adults with unexplained environmental mycobacterial diseases.

Adult↗

PubMed2005

[Is the microscopic morphology of thymoma of clinical significance?].

UNLABELLED: Thymomas are rare mediastinal tumors that have heterogeneous microscopic morphology and uncertain outcome. AIM: The aim of the study was to evaluate the practical usefulness of WHO classification by correlation of histological subtype of thymoma with staging, symptoms and epidemiology. MATERIAL AND METHODS: 87 cases of thymomas was reclassified according to the WHO Histologic classification and Masaoka staging system. Clinical symptoms, age and sex of the patient were taken into consideration in each case. RESULTS: The histological subtypes was diagnosed in order of frequency: AB (n = 21; 24%), B2 (n = 19; 22%), B1 (n = 14; 16%), A (n = 6; 7%), C (n = 4; 5%), B3 (n = 3; 3%). Combined types was observed in 17 (20%) cases. Tendency to invasion increased as follows: A < AB < B1 < B2 < B3 < C. The median value of patients' age was: type A--67,5 years, type AB--62 yrs., type B1--48 yrs., type B2--52 yrs., type B3--32 yrs. and type C (thymic carcinoma)--49,5 yrs. No correlation was found between histological type and sex of patient. The most often clinical symptom--myasthenia, accompanied predominantly B2 thymomas. CONCLUSIONS: The WHO classification is useful in routine pathologic examination of thymomas, because it enables to distinguish histological subtypes with different clinical representation.

Adult↗

PubMed2005

[Exogenous lipid pneumonia--a report of four cases].

Exogenous lipoid pneumonia (ELP) is a rare interstitial pulmonary disease caused by aspiration of the oily material. It is known to mimic many pulmonary diseases and causes many diagnostic difficulties. We present four patients with ELP. The clinical picture was very heterogenous. We described these cases to show that the use of the mineral oils may increase the risk of lipoid pnemonia.

Diagnosis, Differential↗

PubMed2005

[Caplan's syndrome: case report].

A case of a 56-years old male with rheumatoid arthritis and unclear tumoral radiological changes in the lungs was described. Since noninvasive diagnostic procedures failed to explain nature of the pulmonary changes, an open pulmonary biopsy was performed. Pathological examination revealed presence of rheumatoid nodules and pneumoconiosis, typical for Caplan's syndrome. In the past, the patient had been working in foundry industry for 16 years and he had been exposed to silica and iron dust. Articular symptoms were revealed prior to finding the lung changes even dust exposure had occurred many years earlier. In spite of the fact that rheumatoid arthritis is a relatively common disease and pneumoconiosis is also not rare entity, the coexistence of both conditions i.e. Caplan's syndrome has been rarely diagnosed and described.

Arthritis, Rheumatoid↗

PubMed2005

[The significance of the serum tumour markers as a prognostic and predictor factors in nonsmall cell lung cancer patients].

The aim of the study was to assess the role of serum tumour markers (NSE, Cyfra 21-1, CEA, LDH, ferritin) as a prognostic and predictive factors in 79 patients with advanced NSCLC treated with chemotherapy. Objective response to treatment was significantly more frequent in the patient with serum NSE > 12.5 ng/ml. Progression of disease was observed more often in patients with serum Cyfra 21-1 >10 ng/ml or LDH >480 U/L. CEA >3 ng/ml, LDH >480 U/L, for coefficient >1, NSE >20 ng/ml and Cyfra 21-1 >10 ng/ml had a negative impact on survival in univariate analysis. Independent negative prognostic significance of fer coefficient >1 was confirmed by multivariate analysis.

Adult↗

PubMed2005

[Lymphocytic interstitial pneumonia in primary Sjögren syndrome].

Ljmphocytic interstitial pneumonia (LIP) is a rare form of interstitial pneumonia. It can occur as a idiopathic disease however most frequently associated with other diseases, particularly Sjogren syndrome. We present a 58 years old woman, with primary Sjögren syndrome who developed after 7 years interstitial lung disease. LIP was diagnosed on the basis of histological examinations of specimens obtained by open lung biopsy. Diffuse infiltrations composed of small polyclonal lymphocytes, plasma cells with reactive follicules were found. Infiltrates observed in bronchial and bronchiolar walls expanded into interlobular and alveolar septae. Prednisone in a dose of 40 mg per day was introduced and clinical, radiological, spirometric and gasometric improvement was observed. The dose of prednisone was gradually reduced but is still continued.

Collagen Diseases↗

PubMed2005

[Recurrent pneumothorax as alone manifestation of mesothelioma pleurae].

Malignant mesothelioma of the pleura is a rare neoplasm with a poor prognosis. The most often symptoms of this disease are dyspnoe and chest wall pain, which is often associated with pleural effusion. We present 35-years old woman with 5 epizodes of pneumothorax during 3 years (4 of right side and 1-bilateral) in whom bilateral pleurodesis was performed. Specimens of lung and pleurae were examinated. Result of microscopic and immunohistochemical examination was: mesothelioma epithelioides pleurae (mesothelium +, calretyninaa +, EMA+ membrane reaction, desmine +/-).

Adult↗

PubMed2004

[Granular cell tumor--a rare, benign respiratory tract neoplasm in the material of the Institute of Tuberculosis and Lung Diseases].

The granular cell tumor (GCT) is a nodule that arises most commonly in the skin, the breast or the tongue. The vast majority are benign. Approximately 6-10% of granular cell tumors have been reported in the lower respiratory tract. The clinical, pathological and immunohistochemical findings of eleven cases are described in our material consisted of 6 males and 5 females aged from 35 to 58 years (median, 46 years). The GCT were solitary lesions in all our patients. The tumors were located in trachea (6 cases) and in bronchus (5 cases). They were found during bronchoscopy performed because of symptoms of pneumonia, lung cancer and hemoptysis or dyspnea alone. Diameter of the tumors ranged from 0.2-2.5 cm (median 1.2 cm). Six tumors were surgically excised and 5 were endoscopically removed. Pulmonary GCT behave in a benign fashion. It was observed that tumors of less than 8 mm were more amenable to endoscopic removal and larger tumors were more likely to infiltrate through the bronchial wall. Histologically, the GCT showed submucosal infiltrates of round or oval cells with abundant granular cytoplasm. The tumors cells were positive for S-100 protein, neuron specific enolase, CD68 and vimentin. Our immunohistochemical results are consistent with this concept.

Adult↗

PubMed2004

[Hypersensitivity pneumonitis recognised by open lung biopsy in patients at the Institute of Tuberculosis and Lung Diseases].

UNLABELLED: Diagnosis of hypersensitivity pneumonitis (HP) is made on the basis of a combination of clinical features connected with the exposition to organic dusts, radiographic abnormalities, lung function tests and immunological tests. Open lung biopsy (OLB) and histological examination is not necessary and is indicated mainly in chronic and subacute form of HP. In the chronic form of HP symptoms often do not show a temporal relationship with antigen exposure and errors occur especially if specific questions are not asked about exposure to antigen if HP. The aim of this paper was to analyse whether OLB was necessary in all patients in whom HP was recognised just after this procedure. Material included 21 patients in whom microscopic examination suggested HP among 258 pts in whom OLB was performed in the period of 1998-2003. There were 13 men and 8 women at the age of 18 to 65 years, mediana 42 years. RESULTS: We found 3 groups of pts. Group "+" included 9 exposed pts. They were asked and confirmed contact with birds, hay and 7 of them observed correlation between exposition and symptoms. The second Group "-" included 7 pts who were asked about exposition and who answered in the negative. In all of them HRCT scans suggested HP. Restriction was below 70% of VC in 3 pts. The third Group "0" included 5 pts who were not asked about exposition and correlation with symptoms. Two from them lived in the rural area. CONCLUSIONS: Open lung biopsy was not necessary for 6 patients in Group "+", because then had enough symptoms to recognise HP. In 3 patients of Group "+" and in 5 of Group "0" OLB could be not necessary because specific anamnesis and additional examinations were not collected. OLB was necessary method for 7 patients in Group "-".

Adult↗

PubMed2004

[Organizing pneumonia--analysis of 18 own cases].

Organizing pneumonia (OP) is a rarely diagnosed disease, however the incidence ratio was estimated as 6-7/100000. Disease can occur in cryptogenic form or as a secondary reaction to various noxious agents, drugs, and ionising radiation, as a concomitant disease to infections, lympho- and myeloproliferative disorders, and connective tissue diseases. Symptoms of OP are non-specific therefore lung biopsy and histological examination are necessary for diagnosis. Eighteen cases of OP, 15 women and 3 men, aged 40 to 76 years, are presented with analysis of clinicopathological characteristic and therapeutic problems. In all cases diagnosis was confirmed by open lung biopsy. In one case radiotherapy and in one trastuzumab treatment was the cause of OP. In further 3 women antibodies against Chlamydia pneumoniae and in one--against Mycoplasma pneumoniae were found in serum. Probably Hashimoto disease was the cause of one case. In 12 patients the OP was idiopathic. Majority of patients were treated by prednisone (0.5 mg/kg). In one patient regression without any treatment was noticed and in other one--after cessation of trastuzumab. Five women were treated by clarithromycin. In 3 of them regression was observed but in other 2 corticotherapy was necessary. The observation period ranged from 1 month to 9 years, mean 34 months.

Adult↗

PubMed2004

[Solitary fibrous tumor of the pleura in 75-year old woman].

A 75-year-old asymptomatic woman with stable coronary heart disease presented tumor in lower left lobe on routine chest radiograph. A CT scan showed a large sharply delineated mass at this site (84 x 52 x 90 mm). There were no signs of infiltration, no abnormalities were seen in mediastinal structures and on the right side. The pedunculated tumor was resected during left thoracotomy (posterolateral incision). Histological examination revealed spindle-like cells and rich collagen net. Mitoses and necrosis were absent. Final diagnosis was: solitary fibrous tumor of the visceral pleura. During 7-year follow-up recurrence was not observed.

Aged↗

PubMed2004

[Organizing pneumonia--own experiences with clarithromycin treatment].

Organising pneumonia (OP) is a rare syndrome that has been associated with a variety of underlying disorders, including infections, collagen vascular diseases, toxic fumes, cancer, drugs and radiotherapy. Cryptogenic form is also observed. Steroids are usually effective in the treatment of OP, but other treatment regimens have been used as well. We present 5 women with OP, age ranged 57-76 years (mean - 67 years). Two of them were smokers and three were non-smokers. One patient was treated because of hyperthyreosis, one of COPD, and four had a hypertension. Four of them were diagnosed by the open lung biopsy and one by transbronchial lung biopsy. Dyspnoea (100%), cough (100%), fever (80%), weight loss (40%), chest pain (20%), were the most frequently noticed symptoms. All patients had bilateral consolidations with areas of ground glass attenuations at chest x-ray and HRCT. Migratory pattern of them was observed in four patients. Significant elevation of antibodies titers against Chlamydia pneumoniae was revealed in two patients. In all patients clarithromycin in a dose 0.5 g b.d. was administrated. Complete clinical and radiological remission was obtained after 3 months of clarithromycin therapy in 3 patients (one had Chlamydia antibodies). Two patients had not obtained significant improvement during the first two weeks of therapy so prednisolone in a dose 0.5 mg/kg/d was introduced. Also complete remission was noticed in these patients. The observation period ranged from 8 months to 4 years (mean - 34 months). Our study confirms that OP can be treated by the use of clarithromycin. It may be the alternative treatment, particularly for patients in whom probability of adverse reactions in the course of steroid treatment is high.

Aged↗

PubMed2003

[Purulent lung diseases necessitating surgical treatment--case reports].

Majority of purulent lung diseases are treated with antibiotics. But sometimes this therapy is not successful and surgical treatment is necessary. Two patients are described in this paper. 68 years old woman in whom infiltration in left lung enlarged during antibiotics therapy. CT examination did not allow to differentiate between abscessus and tumor. Results of bacteriologic and cytologic examination of transthoracic biopsy were negative. During thoracothomy abscessus was resected and diagnosis was confirmed by patomorphologic examination. The second case: 16-years old girl was addmitted to hospital because of high temperature and abnormal chest X-ray picture (infiltration and liquid level in lower right lobe). During puncture the pus was evacuated but bacteria were not cultured. Antibiotics were not effective. Thoracothomy was done and large cysta bronchogenes was resected. In both cases surgical treatment was effective.

Adolescent↗

PubMed2003

[Eosinophilic myocarditis in allergic angiitis and granulomatosis (Churg-Strauss syndrome) ].

Allergic angiitis and granulomatosis (Churg-Strauss syndrome) is one of the rare form of idiopathic (primary) vasculitis. It is characterized by asthma, blood hypereosinophilia and typical microscopic features: nectrotizing vasculitis, tissue infiltration by eosinophils and extravasal granulomas. We present an autopsy case of 55-years old man with Churg-Strauss syndrome complicated by eosinophilic myocarditis.

Autopsy↗

PubMed2003

[Wegener's granulomatosis overlapped with Takayasu arteritis complicated by thrombosis of the internal jugular vein].

A 30 year-old woman with Wegener's granulomatosis with typical lesions of nose, mouth, skin, lung and positive c-ANCA titre was admitted to our hospital because of ineffective treatment with corticosteroids and cyclophosphamide. Infiltration of the left side of chest wall and collateral circulation were seen during physical examination. Ultrasound examination revealed thrombus in the left internal jugular vein and in the left subclavicular vein. Angio CT revealed parenchymal infiltration in the lower right lobe, thrombi in segmental arteries in this area and enlarged mediastinum. MRI examination revealed thickened aorta wall to 25 mm with its contrast enhancement. It suggested inflammatory infiltration of aorta arcus and its branches. This picture was similar to early stages of Takayasu arteritis but our patient had no typical clinical symptoms related to narrowing or obliteration of large arteries. We recognised Wegener's granulomatosis with overlapped Takayasu arteritis with thrombosis of jugular vein and pulmonary thrombosis. In differential diagnosis: Wegener's granulomatosis with large arteries involvement is taken into account. Treatment with cyclophosphamide and prednisolone was effective.

Adult↗

PubMed2002

Estrogen and progesterone receptors in non small cell lung cancer patients.

The role of sex hormones in the pathogenesis of lung cancer is still unknown. There are conflicting results regarding immunohistochemical detection of the estrogen and progesterone receptors expression in non small cell lung cancer. To clarify these discrepancies 32 samples of lung carcinoma tissues obtained by lobectomy or pneumonectomy were studied. Two monoclonal antibodies (6F11 and ID5) for estrogen receptor detection and one (1A6) for progesterone receptor detection were used. Eighteen adenocarcinoma and 14 squamous cell carcinoma cases were investigated. There were 11 women and 7 men with adenocarcinoma and 4 women and 10 men with squamous cell carcinoma. Weak (+1) nuclear estrogen hormone receptor expression was detected in only one specimen of a woman with adenocarcinoma and in one specimen of a man with squamous cancer. None of the 32 blocks of paraffin embedded specimens expressed progesterone receptor. The positive estrogen and progesterone receptors expression in cancer tissue is an important argument against the pulmonary origin of the unknown primary tumor.

Adenocarcinoma↗

PubMed2002

[Pulmonary actinomycosis as a complication of foreign body aspiration].

This first polish paper presents respiratory actinomycosis as a complication caused by aspiration of a foreign body. Aspiration of a citrus fruit stone occurred as a result of esophagus stenosis and dysphagia caused by a mistake made by a blind person of drinking a caustic substance. Clinical and bronchoscopic features suggested bronchogenic carcinoma but it's not confirmed by two histopathologic examinations of section from pathological bronchial changes. Diagnosis was based on the morphological picture of sections taken during third fibreoptic bronchoscopy and on cytological sputum examination. Removing the aspired foreign body from bronchus and prolonged penicillin treatment resulted in clinical and radiological amelioration.

Actinomycosis↗

PubMed2002

[A case of multiple pulmonary hyalinizing granuloma].

Pulmonary hyalinizing granulomata are unusual, noninfectious lesions of the lung of uncertain etiology. The pathologic entity may represent a peculiar form of pulmonary immune reaction. We report the history of a 23-year-old woman who had nodular lesions in the right lung without any other abnormalities. Non-invasive procedures didn't yield diagnosis. Thoracotomy was performed. During intraoperative microscopic examination of one of 4 nodules, neoplastic disease was excluded. Remaining 3 nodules were also resected. The pulmonary hyalinizing granuloma was recognised in the histological examination. Follow-up after 12 months didn't reveal recurrence.

Adult↗